Woman fights disease that took everything

Part II: Huntington's disease killing O.C. family. Yesterday:
Frances Saldaña unknowingly marries into a family that has the Huntington's gene and bears three children. She sees her husband,
Hector Portillo, die as a result of it in 1989.

H
ector's and Frances' three children each stood a 50 percent chance of inheriting their father's HD gene. “They would ask me once in awhile, ‘Am I going to have dad's disease?' ” Frances recalls. “I would say, ‘No, no, you look more like me.' ”

Frances knew that physical appearance had no bearing on whether they inherited the deadly gene, but she didn't want her offspring to live a childhood of constant fear. The signs of Huntington's disease usually don't start to appear until victims are in their 30s or 40s.

That was the case with Hector, but it isn't always so. In the case of
Marie, their youngest daughter, Frances saw warning signs – decreasing motor skills – when the girl was 11 or 12.

A research team that included UC Irvine's
Leslie Thompson had isolated the HD gene in 1993, and a test for it became available during Marie's childhood. When she turned 18, she went in for the test, which confirmed she had HD.

As Marie's condition deteriorated, Frances put her in a board-and-care home. To make the situation even more tragically bizarre, Marie's roommate turned out to be the elderly woman who had struck Hector with her car as he awkwardly tried to cross the street one day. By the time Frances realized the connection, the woman and her daughter had grown very close, so Frances never told either. Marie died in 2009, never knowing that one of her best friends had been the woman who had accidentally killed her father.

By 1995,
Margie, Frances' and Hector's oldest child, had not shown signs of the disease, but she wanted to get married, so she went in to be tested. She too was positive. She and her fiancé decided to marry anyway. She started showing HD symptoms a few years later but did not become completely incapacitated until last year. Her husband,
Craig, cares for her in their home.

“A lot of husbands walk away. A lot of wives walk away. But he's there for her every day,” Frances says of her son-in-law.

Frances thinks she saw behavioral signs of HD in her son,
Michael, when he was still in his teens. He was uncontrollable. At 16, he took off for Paris on his own to attend cooking school. He was fanatically focused on becoming a chef and opening a fancy restaurant in Manhattan.

“He lived fast and furious, traveled all over and did risky things.” She wondered whether it was the knowledge that he might have HD that pushed him or whether it was the HD itself. As his handwriting started to falter and he became more estranged, she realized he had the disease.

One day in 2002, she got a phone call from the owner of a 7-Eleven in Seattle. Michael, 32, had been sitting outside his store all day. He had been mugged for his money and luggage. He was helpless. It was time. She placed him in a board-and-care home in L.A., where for six years she drove up several times a week to see him. A few weeks ago, she was finally able to place him in a facility in Santa Ana where she can see him every day.

Two weeks ago, I went with Frances to his room, where he lay in a bed surrounded by heavy cushions so he doesn't hurt himself. He was alert but largely uncommunicative. He did smile at his mother, however, and when she reached out her palm and said, “High five!” he responded by slapping it with his. He can no longer eat solid food.

“I think he is at the end-of-life stage,” she told me later. “I can feel it. He is sleeping a lot.”

The have odds beat Frances, badly. In theory, only one or two of her children should have inherited the gene. But all three did. She has watched her husband and their youngest child die. Now she is watching the two oldest die. While she is doing so, however, she is trying to fight the disease as best she can.

She took a job in administration at UCI so she could be where the research for a cure was happening. She started volunteering to talk to medical students about HD so “they can understand what they are dealing with.” Active for years in the Huntington's Disease Society of America, she decided to resign last year and start a purely local group.

She and two other women,
Linda Pimental and
Jean Abdalla, founded HD-CARE (Huntington's Disease Community Advocacy Research & Education), which will send 100 percent of its fundraising proceeds to fund the UCI research. It launches April 20 with “Sounds by the Sea – A Chocolate, Wine & Jazz Experience” from 4 to 7 p.m. at the Hotel Laguna. Information is available at
hdcare.org.

“I just don't want anyone to go through what my husband went through, what my children went through – waking up every day with this sense of impending doom. My hope is that there is a cure within my lifetime.”

Mickadeit writes Mon.-Fri. Contact him at 714-796-4994 or fmickadeit@ocregister.com

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